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The classification of primary cutaneous lymphomas and lymphoproliferative disorders (LPD) is continuously evolving by integrating novel clinical, pathological and molecular data. Recently two new classifications for haematological malignancies including entities of cutaneous lymphomas were proposed: the 5th edition of the WHO classification of haematolymphoid tumours and the International Consensus Classification (ICC) of mature lymphoid neoplasms. This article provides an overview of the changes introduced in these two classifications compared to the previous WHO classification. The main changes shared by both classifications include the downgrading of CD8+ acral T‐cell lymphoma to CD8+ acral T‐cell LPD, and the recognition of entities that were previously categorized as provisional and have now been designated as definite types including primary cutaneous small or medium CD4+ T‐cell LPD, primary cutaneous gamma/delta T‐cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T‐cell lymphoma, Epstein–Barr virus‐positive mucocutaneous ulcer. Both classifications consider primary cutaneous marginal zone B‐cell clonal neoplasm as an indolent disease but use a different terminology: primary cutaneous marginal zone lymphoma (WHO) and primary cutaneous marginal zone LPD (ICC). The 5th WHO classification further introduces and provides essential and desirable diagnostic criteria for each disease type and includes chapters on reactive B‐ or T‐cell rich lymphoid proliferations formerly referred as cutaneous pseudolymphomas, as well as histiocyte and CD8 T‐cell rich LPD in patients with inborn error of immunity. As already emphasized in previous lymphoma classifications, the importance of integrating clinical, histological, phenotypic and molecular features remains the crucial conceptual base for defining cutaneous (and extracutaneous) lymphomas. [ABSTRACT FROM AUTHOR]

Background: Primary cutaneous B-cell lymphomas (CBCL) are a group of rare malignant skin diseases that represent approximately 20%--30% of all primary cutaneous lymphomas (PCL). Previous studies revealed impaired health-related quality of life (HRQoL) in patients diagnosed with primary cutaneous T-cell lymphoma (CTCL). Currently, only small-sized studies investigated HRQoL in CBCL patients and lacked detailed analysis of respective subtypes. Objectives: This study aims to investigate HRQoL in CBCL patients to identify independent factors of HRQoL impairment in CBCL patients. Methods: One hundred CBCL patients were recruited from eight German PCL centres in this multicentric, cross-sectional study from 2021 to 2022. The patients completed the dermatologic HRQoL questionnaire Skindex-29 and an investigator-designed 'CBCL-Questionnaire' with additional questions on HRQoL and clinical characteristics. Results: The Skindex-29 revealed that HRQoL in CBCL patients is impaired on a mild to moderate level. The multiple regression analysis identified parameters like worries about dying, feeling prejudiced/discriminated and impairment of daily activities to be independently associated with impairment of HRQoL. Highest scores for HRQoL impairment were found in patients with primary cutaneous follicle centre lymphoma while on rituximab treatment and in patients with primary cutaneous marginal zone lymphoma while on watchful waiting. Conclusions: HRQoL is impaired in CBCL patients, even though, in the face of indolent disease course and favourable prognosis in the majority of cases. Of note, our investigator-designed tool identified worries about dying, feeling prejudiced/discriminated, and the type of treatment to have a negative impact on patients' HRQoL. Our study highlights the importance of a thorough patient--doctor communication to capture overall disease burden because generic HRQoL tools might lack of disease-specific items. [ABSTRACT FROM AUTHOR]

Michael Tronnier, Christina Mitteldorf Department of Dermatology, Klinikum Hildesheim GmbH, Hildesheim, Germany Abstract: Whereas thin melanomas have an excellent prognosis after sufficient surgical treatment, melanoma disease in advanced stages is still a therapeutic challenge. After decades of frustrating studies, new therapeutic strategies have come up in the past few years. On the one hand, increasing insights into the molecular aberrations in melanoma have led to specific "targeted" therapies to affect only the mutated tumor cells, as in many other types of cancers. Today there are few "targeted" substances which are already approved and successfully used for single or combination therapy, but many others are under development. While on the other hand, nonpersonalized strategy substances have been developed successfully inducing an immunologic tumor response. Both kinds of therapy have been found to result in an improvement not only of the response rate, but also of the overall survival in metastatic disease, which represents a milestone in melanoma therapy. However, using these therapies there is still much to learn regarding the effects, the side effects, and the limitations of these promising substances. Keywords: melanoma, treatment, targeted therapy, immunotherapy, BRAF, CTLA-4

I Dear Editor i , Desmoplastic melanoma (DM) is a rare melanoma subtype, which often shows perineural infiltration.1,2 Clinically, it presents with unremarkable, scar-like, amelanotic patches or plaques, and is clinically often not recognizable as a melanocytic tumour.3,4 This leads to late diagnosis going along with high Breslow thickness and advanced tumour stage.5 There is a strong association with sun exposure, the most common localization is in the head and neck region and it occurs mostly in elderly patients.6 Desmoplastic melanoma metastasizes less frequently into the lymph nodes5 and melanoma-specific survival appears to be longer than for other types of melanoma.6,7 However, local recurrences are more frequent because of prevalent perineural spread5 and often difficult complete excision.7 Neuropathy has rarely been reported and then mostly in advanced tumour recurrences.8 In any case, sensory deficits and paresis of peripheral nerves have not been reported as early prodromal signs of DM. 2c, inset), SOX (Sry-related HMg-Box gene)-10 and PRAME (PReferentially expressed Antigen in MElanoma), but negative for MelanA (Melanoma Antigen Recognized by T cells-1, MART-1) and HMB (Human Melanoma Black)-45. Neurotropic melanoma: a variant of desmoplastic melanoma. [Extracted from the article]

Background: Cutaneous peripheral T‐cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm. Objectives: The European Organization for Research and Treatment of Cancer cutaneous lymphoma taskforce (EORTC CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyse their clinical, histological, immunophenotypic features and outcome. Methods: Retrospective analysis of clinical data and histopathological features by an expert panel. Results: Cutaneous PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumours or disseminated papulo‐nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3+/CD4−/CD8− and CD3+/CD4+/CD8+. Moreover, 18% of the cases exhibited an aberrant expression of the B‐cell marker CD20 by the tumour cells. All solitary tumours were located on the limbs and presented a high expression of GATA‐3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow‐up of 36 months (mean value; range 3–144). The survival rates were 61% after 3 years (CI, 43–85%) and 54% after 5 years (CI, 36–81%). Small to medium‐sized morphology of tumour cells was associated with a better outcome than medium to large or large tumour cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA‐3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first‐line treatment for solitary lesions. Conclusions: Cutaneous PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS. [ABSTRACT FROM AUTHOR]

Background: Deep learning convolutional neural networks (CNN) may assist physicians in the diagnosis of melanoma. The capacity of a CNN to differentiate melanomas from combined naevi, the latter representing well‐known melanoma simulators, has not been investigated. Objective: To assess the diagnostic performance of a CNN when used to differentiate melanomas from combined naevi in comparison with dermatologists. Methods: In this study, a CNN with regulatory approval for the European market (Moleanalyzer‐Pro, FotoFinder Systems GmbH, Bad Birnbach, Germany) was used. We attained a dichotomous classification (benign, malignant) in dermoscopic images of 36 combined naevi and 36 melanomas with a mean Breslow thickness of 1.3 mm. Primary outcome measures were the CNN's sensitivity, specificity and the diagnostic odds ratio (DOR) in comparison with 11 dermatologists with different levels of experience. Results: The CNN revealed a sensitivity, specificity and DOR of 97.1% (95% CI [82.7–99.6]), 78.8% (95% CI [62.8–89.1.3]) and 34 (95% CI [4.8–239]), respectively. Dermatologists showed a lower mean sensitivity, specificity and DOR of 90.6% (95% CI [84.1–94.7]; P = 0.092), 71.0% (95% CI [62.6–78.1]; P = 0.256) and 24 (95% CI [11.6–48.4]; P = 0.1114). Under the assumption that dermatologists use the CNN to verify their (initial) melanoma diagnosis, dermatologists achieve an increased specificity of 90.3% (95% CI [79.8–95.6]) at an almost unchanged sensitivity. The largest benefit was observed in 'beginners', who performed worst without CNN verification (DOR = 12) but best with CNN verification (DOR = 98). Conclusion: The tested CNN more accurately classified combined naevi and melanomas in comparison with trained dermatologists. Their diagnostic performance could be improved if the CNN was used to confirm/overrule an initial melanoma diagnosis. Application of a CNN may therefore be of benefit to clinicians. Linked Commentary: M.V. Heppt et al. J Eur Acad Dermatol Venereol 2020; 34: 1134–1135. https://doi.org/10.1111/jdv.16577. [ABSTRACT FROM AUTHOR]

Dear Editor, CD30, also known as tumour necrosis factor receptor 8 (TNF-R 8), was initially called Ki-1 and is a cell-surface molecule that was first described by a group of pathologists from Kiel in 1982.1 CD30 can be targeted with the drug brentuximab vedotin (BV), which is approved for relapsed or refractory CD30 SP + sp cutaneous T-cell lymphoma (CTCL) including primary cutaneous anaplastic large-cell lymphoma and CD30 SP + sp mycosis fungoides and Sézary syndrome. The question of how much CD30 expression is needed for a valuable clinical response to a CD30-targeted therapy is still not finally resolved. [Extracted from the article]

6 Parodi A, Micalizzi C, Rebora A. Intralesional natural interferon alpha in thetreatment of Crosti's lymphoma (primary cutaneous B follicular centre-cell lymphoma): report of four cases. Primary cutaneous follicle centre lymphoma, spindle cell type, presenting with multicentre figurated erythema and complete remission after intralesional injections of ultra-low-dose Interferon alpha-2a Spindle-cell cariants of primary cutaneous follicle center B-cell lymphomas are germinal center B-cell lymphomas by gene expression profiling using a formalin-fixed paraffin-embedded specimen. [Extracted from the article]

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Copyright of Der Pathologe is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

We report a patient with lip enlargement due to specific infiltrates of marginal zone lymphoma. His medical history included a B-cell chronic lymphatic leukaemia (B-CLL), which was diagnosed 10 years ago based on examination of peripheral blood cells and clinically evident generalized swelling of lymph nodes. The patient declined both lymph node and bone marrow biopsy. [Extracted from the article]

Copyright of Der Hautarzt is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)